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Table of Contents    
LETTER TO EDITOR
Year : 2013  |  Volume : 61  |  Issue : 6  |  Page : 687-690

Ependymoma: Unusual differential for a totally extramedullary intraspinal tumor


1 Department of Neurosurgery, John H. Stroger Jr. Hospital of Cook County, Chicago, IL, USA
2 Department of Neurosurgery, RUSH University Medical Center, Chicago, IL, USA

Date of Submission24-Oct-2013
Date of Decision18-Nov-2013
Date of Acceptance18-Feb-2013
Date of Web Publication20-Jan-2014

Correspondence Address:
Manish K Kasliwal
Department of Neurosurgery, RUSH University Medical Center, Chicago, IL
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.125386

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How to cite this article:
Gardner L, Kasliwal MK, Hempeck N, Utset M, Gandhi YN. Ependymoma: Unusual differential for a totally extramedullary intraspinal tumor. Neurol India 2013;61:687-90

How to cite this URL:
Gardner L, Kasliwal MK, Hempeck N, Utset M, Gandhi YN. Ependymoma: Unusual differential for a totally extramedullary intraspinal tumor. Neurol India [serial online] 2013 [cited 2019 Sep 18];61:687-90. Available from: http://www.neurologyindia.com/text.asp?2013/61/6/687/125386


Sir,

Benign nerve sheath tumors (NST) or meningiomas are the most common intradural extramedullary (IDEM) tumor in adults. [1] On the contrary, ependymoma is the most common primary intramedullary spinal tumor in adults. [2] Although the myxopapillary variant of ependymoma arising from the filum is extramedullary, IDEM location of this glial lesion outside filum is exceptionally rare. [3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19]

A 27-year-old female patient presented with 2 years history of worsening band-like sensation across the chest and difficulty ambulating with urinary symptoms for 2 months with neurological examination consistent with thoracic myelopathy. Magnetic resonance imaging (MRI) demonstrated presence of a long segment IDEM lesion from T2 to T7 with diffuse enhancement and cystic component displacing the spinal cord to the left [Figure 1]. With a possible diagnosis of NST, a T2-T8 laminoplasty was performed. Intraoperatively, a soft-tissue tumor was seen with the spinal cord displaced but not infiltrated by the tumor. The tumor was totally extramedullary and could be dissected from the spinal cord permitting gross total resection. Histopathology confirmed the diagnosis of an ependymoma [Figure 2]. Post-operative MRI showed no residual tumor with imaging of the neuraxis demonstrating no other synchronous lesion [Figure 3]. The patients neurological symptoms resolved completely at her last follow-up at 8 months. Radiation therapy was withheld in view of complete resection but she has been kept under clinical surveillance in view of the diagnosis of ependymoma.
Figure 1: Magnetic resonance imaging (MRI) of the thoracic spine showing a long segment intradural extramedullary lesion from T2 to T7 with diffuse enhancement and cystic component displacing the spinal cord to the left. Left: Sagittal T1W MRI with contrast, middle: Axial T1W MRI with contrast and right: T2W sagittal MRI

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Figure 2: Densely cellular glial neoplasm with abundant perivascular pseudorossettes characteristic of ependymoma

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Figure 3: Sagittal T1W, T1W with contrast and T2W magnetic resonance imaging showing no obvious residual tumor with post-operative changes and well decompressed spinal cord

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Intraspinal tumor in adults can be intramedullary or IDEM. The diagnosis of IDEM tumors often connotes good prognosis as majority of these tumors are benign with excellent clinical outcome. Spinal ependymomas have been described exceptionally rare as extramedullary and only seventeen cases have been described so far as per our review of the literature [Table 1]. The plausible explanation for an IDEM ependymoma comes from the embryologic theory according to which a collection of ependymal cells are not contained within the anatomic central canal location of the spinal cord during neural tube allowing ectopic cells to be within the IDEM space after birth. [10],[11] Review of the literature shows that these tumors are usually benign and slow growing, with subtle symptoms present over a year before diagnosis. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[14],[15],[16],[17],[18],[19],[20],[21] The treatment of choice is total resection, with radiation usually reserved for cases with incomplete resection and/or recurrence. [5],[8],[15],[16] Although radiotherapy has been proposed to prolong local control and survival after incomplete resection, [22] histological grade and surgical extent of resection remains the most important independent predictors of longer progression-free survival. [23] Nevertheless, even Grade II ependymomas may recur after complete excision at site different from the primary, as Robles, et al.[15] reported a recurrence of extramedullary ependymoma distal to the prior surgery site implicating that these tumors may not be benign as are usually thought and the natural history of other benign IDEM like NST and meningiomas cannot be extrapolated to extramedullary ependymomas. This highlights the importance of close imaging surveillance for these patients and though not conclusively proven, the possible role of avoiding tumor dissemination and spillage during surgery in cases of possible IDEM ependymoma to avoid recurrences at different CNS location. [15],[16] Though, it can be argued that the treatment of choice for all IDEM tumors is total resection; in recurrences/subtotal resections for benign tumors like meningiomas and NST, patients generally do not require spinal radiation, unlike ependymomas which may need post-operative radiation, if not excised completely or even upfront if they are Grade III. [10],[11],[13],[16],[22]
Table 1: Details of patients with intradural extramedullary ependymoma reported in the literature

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Though, IDEM ependymoma generally have a good neurologic recovery after complete resection; possibility of potential recurrence, distant metastasis due to CSF dissemination and anaplastic transformation should be watched for and these patients should be kept under close clinical and radiological surveillance. Awareness of this tumor as a differential of IDEM neoplasm with its possible clinical behavior may help pre-operative patients counseling and appropriate management.

 
  References Top

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11.Kinsman MJ, Callahan JD, Hattab EM, Cohen-Gadol AA. Extramedullary spinal ependymoma: A diagnostic challenge and review of the literature. Clin Neurol Neurosurg 2011;113:661-4.  Back to cited text no. 11
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12.Li MH, Holtås S, Larsson EM. MR imaging of intradural extramedullary tumors. Acta Radiol 1992;33:207-12.  Back to cited text no. 12
    
13.Oliver B, de Castro A, Sarmiento MA, Argüello C, BlaŸquez MG. Dorsal extramedullary ependymoma (author′s transl). Arch Neurobiol (Madr) 1981;44:215-24.  Back to cited text no. 13
    
14.Payer M, Yonekawa Y, Imhof HG. Solitary thoracic intradural extramedullary ependymoma. J Clin Neurosci 1999;6:344-45.  Back to cited text no. 14
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15.Robles SG, Saldaña C, Boto GR, Martinez A, Zamarron AP, Jorquera M, et al. Intradural extramedullary spinal ependymoma: A benign pathology? Spine (Phila Pa 1976) 2005;30:E251-4.  Back to cited text no. 15
    
16.Schuurmans M, Vanneste JA, Verstegen MJ, van Furth WR. Spinal extramedullary anaplastic ependymoma with spinal and intracranial metastases. J Neurooncol 2006;79:57-9.  Back to cited text no. 16
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17.Son DW, Song GS, Han IH, Choi BK. Primary extramedullary ependymoma of the cervical spine: Case report and review of the literature. J Korean Neurosurg Soc 2011;50:57-9.  Back to cited text no. 17
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18.Wagle WA, Jaufman B, Mincy JE. Intradural extramedullary ependymoma: MR-pathologic correlation. J Comput Assist Tomogr 1988;12:705-7.  Back to cited text no. 18
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19.Wolfla CE, Azzarelli B, Shah MV. Primary extramedullary ependymoma of the thoracic spine. Case illustration. J Neurosurg 1997;87:643.  Back to cited text no. 19
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20.Lee CH, Moon KY, Chung CK, Kim HJ, Chang KH, Park SH, et al. Primary intradural extramedullary melanoma of the cervical spinal cord: Case report. Spine (Phila Pa 1976) 2010;35:E303-7.  Back to cited text no. 20
    
21.Scotti G, Scialfa G, Colombo N, Landoni L. MR imaging of intradural extramedullary tumors of the cervical spine. J Comput Assist Tomogr 1985;9:1037-41.  Back to cited text no. 21
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22.Lin YH, Huang CI, Wong TT, Chen MH, Shiau CY, Wang LW, et al. Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol 2005;71:205-10.  Back to cited text no. 22
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23.Lee SH, Chung CK, Kim CH, Yoon SH, Hyun SJ, Kim KJ, et al. Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: A retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro Oncol 2013;15:921-9.  Back to cited text no. 23
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
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